Anaesthetic Management for Cataract Surgery in VACTERL Syndrome Case Report

Summary V: Vertebral anomalies, A: Anal malformation, C: Cardiovascular defect, TE: Trachealand esophageal malformation, R:Renal agenesis, L: Limb anomalies. underwent cataract surgery under general anaesthesia. She had multiple congenital anomalies like esophageal atresia, imperforate anus (corrected), single kidney & radial aplasia. Anticipating problems of gastro -esophageal reflux & chronic renal failure, successful management was done.


Introduction
VATER Association is a set of congenital malformations occurring in different combinations (at least two). These present asmajor functionalimpairment and appear during first year of life. 1 VATER is an acronym or abbreviation representing the first letter of each feature in association. Because of somenon-causalmalformations whether this pattern is sequence or syndrome is stillunknown. For such situations the word association has been coined. The VATER has been now abbreviated as VACTERL.
[V:Vertebralanomalies, A:Anal malformation, C: Cardiovascular defect 2 , TE:Tracheal and esophageal malformation, R:Renal agenesis 3 , L:Limb anomalies.] This malformationpredominantly occurs in association and with sporadic presentation in families without previous history. 4 Here we report the anaesthetic management of a rare case of VACTERLsyndrome for cataract surgery.

Case report
Eight year old girl, weighing 14kgpresented with diminished vision for six months. Cataract was diagnosed in both the eyes. She was full term normally delivered child, detected to have esophageal atresia & imperforate anus after fewhours of birth. She also had absent radius.Asinglekidney wasaccidentally detected on sonography.
At 30hours oflife she had undergone feeding gastrostomy, colostomy & esophageal pouch was created. At the age of 1½ years, she had undergone anal pull through surgery & colostomy closure was done.At the age of 2 years, esophageal atresia was corrected by formation of tube ofcolon anastomosingwith stomach; subsequently esophagealpouch wasclosed. At the age of 6 years she presented with anuria & diagnosed to have renalhypertension and chronic renalfailure due to single kidney. She wason hemodialysisinitially once a weeknow twice a week. Prior to the day of surgery, she had undergone hemodialysis. She was on oral amlodepin 5mg BD & Losartan 50mg ½ HS since 2 years. She haddeafness since 8 months, using hearing aids and now presented with diminution of vision of both the eyes, diagnosed as bilateral cataract.
On clinicalexamination, (Fig.1-4) she was malnourished,weighing 14Kg& height of 100cm.She was deaf,non-cooperative andunable to stand dueto weakness. Her leftarm was short dueto Radialaplasia. Right arm & forearm had AV fistula. Her pulse rate was 120/ min regular; blood pressure 160/100mmHg measured on left upper arm and body temperature was normal. She was pale, no icterus or cyanosis was observed. Her spine was normal. In cardiovascular system, S1\S2 was normalwith soft systolic murmer. On respiratory system examination, air entry was equal on both the sides; there were no foreign sounds. Mallampati classification could not be elicited, as child was un-cooperative.

Anaesthesia management
Patient was classified asASA grade IV in view of multiple anomaliesand chronicrenalfailure.Patient was dialysed day prior to the surgery & blood investigations weredone. Patient was anoperated caseof esophageal atresia, in which tube of colon was connected from esophagus to stomach. In this operated patient since esophageal sphincter does not exists, so there is always risk of regurgitation. Anticipatingthe risk of regurgitation, patient was kept nil per orally for 12hrs. Antacid (Tab. ranitidine 2-4mg.Kg -1 , 1/4 th tablet) and anti-hypertensive drugs were given with sips of water two hrs before surgery. Peripheral IV cannulation with 22gcannula was done, DNSinfusion was started. Highrisk consent was obtained and patient shifted to operation theatre. Nasogastric tube could not be passed, as child was uncooperative. Cardioscope, pulse oxime-ter, NIBPwere attached to the patient. Glycopyrrolate 60mcg & ondansetron 1mg was given IV. She was preoxygenated with 100% oxygen for 5min, after that midazolam 0.4mg, tramadol 30mgIV were given. Patient was induced with thiopental 100mg IV & atracurium 8mg IV was given. At the time of induction through the transparent mask, reflux of regurgitated materialwas seen in oralcavity.Patient was still breathing spontaneously as relaxant effect wasyet tocome & no IPPV was given. Immediately head low was done & suction done with large bore catheter. Under direct laryngoscopy, orotracheal intubation was done with 5 mm I.D. ETT and ventilated with Jackson & Rees circuit with 100% oxygen. Air entry was checked, tube fixed, throat pack kept. Saturation dropped up to 92%, so endotrachealsuction was done with saline, through endotrachealtube, no foreign material was retrieved. Hydrocortisone 20mg & dexamethasone 2.8mg IV were given. Saturation improved upto 98%, there were no foreign sounds on chest auscultation. Then patient was ventilated with oxygen (50%), nitrous (50%) & halothane intermittently. Surgery was allowed to start, as patient was stable maintaining saturation98% -99%. Intra-operatively vitals were stable, all care to avoid hypothermia & fluid over load was taken in view of CRF. Total surgery time was 45min. X-ray chest on table showed clear lung field. After regaining spontaneous respiration, throat pack was removed, suction done & reversed with neostigmine 0.8mg& atropine 0.3mg. Patient was extubated aftershe wasfully awake. Vitals were stable & shifted to PICUfor observations. Post operatively she was kept on antibiotic & humidified oxygen on venti-mask She did not require bronchodilatoror nebulization.
In 1975 VATER was expanded to VACTERL 5 which includes V: Vertebral anomalies 70%, A:Anal anomalies 80%, C: Cardiac problems 53%, TE: Tracheo-esophageal atresia 70%, R: Renal anomalies 53% & L: Limb anomalies 65%. Radial defect is commonly detected at birth so it is mandatory 6 to rule out other anomalies. Sporadic association of specific birth defects 7 characterizesthe VACTERLsyndrome. Quan & Smith first developed the term VATER association in 1973. VATER association is genetically considered a polytopic change, thus related to blastogenesis & differentfrom monotopicchange related to organogenesis 8 .Although the uncertainty aboutmalformation origin, whether genetic or predisposed 9 ,allinvolved structures are genetically normal. Even parts secondarily affectedby them (spinabifida) are genetically normal. 10 Our patient presented with bilateralcataract. She also had single kidney & radialagenesis. In early child hood patient had under gone correction of esophageal atresia where colon was used for reconstruction of esophagus. The disadvantage of this is colon does not have sphincteric action & motility is also slow. So the risk of gastro-esophageal reflux 11,12 is 50%. Threat to the life can occur duringinduction due to reflux of regurgitated particles & soilingof lungsparenchyma. With the anticipation of above problem, patient was kept nil per orally for 12h. Head up was done. Pro kinetic were not given in view of extra-pyramidal side effects. Adequate pre-oxygenation was done. The large bore suction & transparent mask were kept ready.Also vigilant checkwas keptfor foodparticles regurgitatingin mouth. Thus all steps to avoid aspiration of food particle in lungs were done. With this we could avoid soiling of thelungs. Butsituation may not be the samealways. To avoid the risk of aspiration rapid sequence induction with suxamethonium is an ideal technique. Our patient was the case of CRF ondialysis withmultiple congenital defects & muscle weakness so we could not use suxamethonium,due topotential riskof hyperkalemia leading to cardiac arrest. In these patients care must be takento avoid hyperkalemia, fluid overload, hypothermia. Also due care of AV fistula is needed to avoid injury.Abnormalities in bleeding profile& coagulation can lead to intra-ocular bleeding, as the patient was on dialysis. Patient was anaemic, however she had lost her vision so the riskwas taken to improve her vision. Afterexplainingtheanaesthesia riskto the relatives with informed consent patient was accepted for surgery.
Patient with tracheo-esophageal fistula has susceptibility to respiratory infections due to weakness in tracheal muscles & hyper reactive airways. These patients need active respiratory care with physiotherapy & antibiotics, pre & post operatively. When the colon is used for anastomosis, since esophageal sphincter does not exists, there is always riskof regurgitation. A silent regurgitation is a real threat to life at induction of anaesthesia even if patientis adequatelystarved. Hence history of regurgitation even at rest after food should be ruled out pre-operatively, on repeated enquiry relatives came out with this history in our patient. This demands vigilance & extra care to avoid aspiration.
In conclusion the pre-existinganomalies with potential risk of regurgitation in patient with VACTERL syndrome demands careful pre-operative assessment and use of skillful anaesthetic technique to avoid fatal complications.